Nokea
A 36-year-old woman presented with weakness, lassitude, and feeling easily tired. Her bone marrow aspirate showed 15% myeloblasts and reduced erythropoiesis. The most likely cause is
- A. Acute myeloid leukemia
- B. Acute lymphoid leukemia
- C. Myelofibrosis
- D. Myelodysplastic syndrome
Correct Answer: D
Rationale: The correct answer is D: Myelodysplastic syndrome. The patient's symptoms of weakness, lassitude, and reduced erythropoiesis along with increased myeloblasts in bone marrow are indicative of a myelodysplastic syndrome. This condition is characterized by ineffective hematopoiesis leading to cytopenias and an increased risk of progression to acute myeloid leukemia. Acute myeloid leukemia (choice A) typically presents with more aggressive symptoms and higher blast count. Acute lymphoid leukemia (choice B) primarily affects lymphoid cells, not myeloid cells. Myelofibrosis (choice C) is characterized by fibrosis in the bone marrow, leading to extramedullary hematopoiesis, which is not seen in this case.
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A newborn infant develops jaundice on day of life 2. Labs are drawn, and she has a hemoglobin of 7.4 g/dL with a reticulocyte count of 8%. Upon peripheral blood smear review, she is found to have bizarre red cell forms with significant polkilocytosis. Although her parents have normal blood counts, on review of their peripheral blood smears, they both have a moderate number of ovalocytes. Which of the following is the most likely cause of the infant's red cell findings?
- A. She has an autosomal dominant ankyrin mutation from one of her parents causing hereditary spherocytosis.
- B. She has inherited band 3 variants from each parent and will likely need a splenectomy after she turns 5 years old.
- C. She has inherited an alpha-spectrin mutation from both of her parents and may experience an improvement in her anemia over time.
- D. She has inherited a PKLR variant from each parent, and enzyme testing will be consistent with her diagnosis of pyruvate kinase deficiency.
Correct Answer: C
Rationale: The correct answer is C because the infant likely has hereditary elliptocytosis, an autosomal dominant disorder caused by mutations in the alpha-spectrin gene. Ovalocytes and polkilocytosis are characteristic of this condition. Since both parents have ovalocytes, it is likely that the infant inherited mutations from each parent, resulting in a more severe presentation. Anemia in hereditary elliptocytosis can improve over time due to compensatory mechanisms. Choices A, B, and D are incorrect as they describe different genetic disorders (hereditary spherocytosis, hereditary stomatocytosis, and pyruvate kinase deficiency, respectively) and do not match the clinical and family history provided.
A nurse is monitoring a client who is undergoing anticoagulant therapy with heparin. Which of the following findings should the nurse identify as a possible indication of hemorrhage?
- A. Rapid pulse
- B. Yellowing of the sclera
- C. Elevated blood pressure
- D. Pale-colored stools
Correct Answer: A
Rationale: The correct answer is A: Rapid pulse. When a client is undergoing anticoagulant therapy with heparin, the nurse should monitor for signs of hemorrhage. A rapid pulse can indicate internal bleeding, a common complication of anticoagulant therapy. Other choices such as B: Yellowing of the sclera, C: Elevated blood pressure, and D: Pale-colored stools are not typically associated with hemorrhage. Yellowing of the sclera may indicate jaundice, elevated blood pressure may be related to other conditions, and pale-colored stools may indicate liver or gallbladder issues. Rapid pulse is the most pertinent finding related to hemorrhage in this context.
A nurse works in a gerontology clinic. What age-related change(s) related to the hematologic system will the nurse expect during health assessment? (Select all that apply.)
- A. Dentition deteriorates with more cavities.
- B. Nail are stron and smooth
- C. Progressive loss or thinning of hair occurs.
- D. Sclerae begin to turn yellow or pale.
Correct Answer: C
Rationale: Rationale:
C: Progressive loss or thinning of hair occurs in the geriatric population due to age-related changes in the hair follicles. As individuals age, the hair follicles shrink, leading to hair thinning or loss.
Incorrect Choices:
A: Dentition deteriorating with more cavities is related to oral health, not the hematologic system.
B: Strong and smooth nails are not directly related to age-related changes in the hematologic system.
D: Sclerae turning yellow or pale is indicative of potential liver dysfunction, not a direct age-related change in the hematologic system.
You examine a 10-year-old boy with severe aplastic anemia. He has no dysmorphic features and is at the 50th percentile for height and weight. Family history includes a sister with aplastic anemia unresponsive to anti-human thymocyte globulin (ATG) and cyclosporine who died early in the course of an unrelated donor hematopoietic stem cell transplant complicated by severe mucositis and transplant-related organ toxicities. There are no other siblings. A cousin died of acute myeloid leukemia at age 5 years. A peripheral blood sample test for Fanconi anemia is negative with no increased chromosomal breaks in response to diepoxylbutane or mitomycin C. Which of the following is the most important next step in management?
- A. Administer ATG and cyclosporine.
- B. Search for a donor for matched unrelated transplant.
- C. Send a bone marrow aspirate for Fanconi anemia testing.
- D. Send a skin fibroblast culture for Fanconi anemia testing.
Correct Answer: D
Rationale: The correct answer is D: Send a skin fibroblast culture for Fanconi anemia testing. This is the most important next step in management because the patient's history, including a family member with aplastic anemia and a cousin with leukemia, raises suspicion for a genetic disorder like Fanconi anemia. Testing skin fibroblasts for Fanconi anemia can help confirm or rule out this diagnosis. Administering ATG and cyclosporine (choice A) may not be effective if the underlying cause is a genetic disorder. Searching for a donor for matched unrelated transplant (choice B) is premature without confirming the diagnosis. Sending a bone marrow aspirate for Fanconi anemia testing (choice C) may not yield accurate results as the peripheral blood sample test was negative, making skin fibroblast culture the preferred choice.
A 14-year-old Syrian male with beta thalassemia major has relocated to your community as a refugee. He has been receiving chronic transfusion therapy in Turkey for the past 3 years. On his first visit, you notice that his height is below the fifth percentile. He has skin discoloration and hepatosplenomegaly. His mother reports they have not had regular access to chelation therapy. Laboratory testing shows a serum ferritin of 6,200 ng/mL. A cardiac MRI shows grossly normal cardiac function but a T2* value of 9 ms. What is the most likely cause of his short stature?
- A. Lack of regular blood transfusion causing growth failure
- B. Cirrhosis and liver failure
- C. Ineffective erythropoiesis and chronic anemia
- D. Growth hormone deficiency due to iron deposition in the pituitary
Correct Answer: D
Rationale: The correct answer is D: Growth hormone deficiency due to iron deposition in the pituitary. Iron deposition in the pituitary gland can lead to impaired production and secretion of growth hormone, resulting in short stature. This is a known complication of beta thalassemia major. The patient's elevated serum ferritin levels and T2* value of 9 ms indicate iron overload, which can cause damage to organs including the pituitary gland. Choices A, B, and C are incorrect because the patient's growth failure is primarily due to the impact of iron deposition on the pituitary gland rather than lack of transfusion, cirrhosis, or ineffective erythropoiesis. Regular chelation therapy is essential to prevent iron overload and its complications in patients with beta thalassemia major.